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Myelofibrosis

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https://www.readbyqxmd.com/read/29667765/molecular-profile-of-inflammatory-and-megakaryocytic-factors-in-pediatric-myelodysplastic-syndrome-with-acute-myelofibrosis
#1
Kais Hussein, Meinolf Suttorp, Angelika Stucki-Koch, Irith Baumann, Charlotte M Niemeyer, Hans Kreipe
Pediatric fibrotic myelodysplastic syndromes (ped-MDS-MF) and pediatric primary myelofibrosis (ped-PMF) are rare, and the molecular changes which mediate fibrosis have never been investigated. Histology and gene expression profile of 119 fibrosis/angiogenesis/inflammation/megakaryopoiesis-related factors in bone marrow biopsies were performed (two ped-MDS-MF and one ped-PMF). In one progressive ped-MDS, comparison of MF grade 0 (no myelofibrosis) and MF grade 2 (dense network of reticulin fibres) after 4 months showed that expression of fibrosis-related transcripts increased and dysplastic megakaryocytes formed a dense net of CD42b+ proplatelets...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29665937/-current-understanding-of-myeloproliferative-neoplasm-related-gene-mutations-and-cytokine-review
#2
Zhi-Peng He, Yong Wu
Myeloproliferative neoplasm(MPN) is clonal hematopoietic stem cell disorder characterized by abnormal proliferation and expansion of one or more myeloid lineages. BCR-ABL-negative MPN includes polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The mutations of JAK2, CALR and MPL genes are involved in the pathogenesis of MPN that provided a more complete molecular diagnostic standard for MPN. More and more new mutated genes related to prognosis of MPN were discovered in the past few years, at same time it was found that cytokines were also involved in the genesis and development of MPN...
April 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29665657/recent-advances-in-the-diagnosis-and-management-of-primary-myelofibrosis
#3
Katsuto Takenaka, Kazuya Shimoda, Koichi Akashi
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) in which dysregulation of the Janus kinase/signal transducers and activators of transcription (JAK/STAT) signaling pathways is the major pathogenic mechanism. Most patients with PMF carry a driver mutation in the JAK2, MPL (myeloproliferative leukemia), or CALR (calreticulin) genes. Mutations in epigenetic regulators and RNA splicing genes may also occur, and play critical roles in PMF disease progression. Based on revised World Health Organization diagnostic criteria for MPNs, both screening for driver mutations and bone marrow biopsy are required for a specific diagnosis...
April 20, 2018: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29651917/erythematous-skin-lesions-with-necrotic-centers-on-lower-extremities-due-to-the-use-of-ruxolitinib-for-primary-myelofibrosis
#4
Constantin A Dasanu
Ruxolitinib is a small molecule JAK-2 inhibitor approved for the treatment of certain myeloproliferative neoplasms. Ruxolitinib-related skin toxicity is extremely rare. We report herein an unusual erythematous skin eruption with necrotic centers involving lower extremities in a patient with primary myelofibrosis treated with ruxolitinib. Awareness of this unusual skin toxicity with ruxolitinib becomes even more important as JAK-2 inhibition might soon find clinical applications in dermatology.
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29650953/hsp27-is-a-partner-of-jak2-stat5-and-a-potential-therapeutic-target-in-myelofibrosis
#5
Margaux Sevin, Lucia Kubovcakova, Nicolas Pernet, Sébastien Causse, Franck Vitte, Jean Luc Villeval, Catherine Lacout, Marine Cordonnier, Fernando Rodrigues-Lima, Gaétan Chanteloup, Matthieu Mosca, Marie-Lorraine Chrétien, Jean Noël Bastie, Sylvain Audia, Paul Sagot, Selim Ramla, Laurent Martin, Martin Gleave, Valérie Mezger, Radek Skoda, Isabelle Plo, Carmen Garrido, François Girodon, Aurélie de Thonel
Heat shock protein 27 (HSP27/HSPB1) is a stress-inducible chaperone that facilitates cancer development by its proliferative and anti-apoptotic functions. The OGX-427 antisense oligonucleotide against HSP27 has been reported to be beneficial against idiopathic pulmonary fibrosis. Here we show that OGX-427 is effective in two murine models of thrombopoietin- and JAKV617F-induced myelofibrosis. OGX-427 limits disease progression and is associated with a reduction in spleen weight, in megakaryocyte expansion and, for the JAKV617F model, in fibrosis...
April 12, 2018: Nature Communications
https://www.readbyqxmd.com/read/29650801/the-role-of-jak2-inhibitors-in-mpn-seven-years-after-approval
#6
Francesco Passamonti, Margherita Maffioli
Myeloproliferative neoplasms (MPNs) include essential thrombocythemia, polycythemia vera (PV) and primary myelofibrosis (PMF). Phenotype-driver mutations of JAK2, CALR and MPL genes are present in MPNs and can be variably combined with additional mutations. Driver mutations entail a constitutive activation of the JAK2/STAT pathway, the key signaling cascade in MPNs. Among JAK2 inhibitors (JAKis), ruxolitinib (RUX) has been approved for the treatment of intermediate and high risk myelofibrosis (MF) and of PV inadequately controlled by or intolerant of hydroxyurea...
April 12, 2018: Blood
https://www.readbyqxmd.com/read/29626596/renal-histology-in-a-patient-with-tafro-syndrome-a-case-report
#7
Hiroki Mizuno, Akinari Sekine, Masahiko Oguro, Yoichi Oshima, Masahiro Kawada, Keiichi Sumida, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Rikako Hiramatsu, Eiko Hasegawa, Junichi Hoshino, Naoki Sawa, Takashi Fujii, Kenmei Takaichi, Kenichi Ohashi, Yoshifumi Ubara
An 84-year-old Japanese man was admitted due to anasarca, thrombocytopenia, systemic inflammation, and progressive renal insufficiency, resistant to diuretics, glucocorticoid therapy and plasma exchange. Renal biopsy showed diffuse endocapillary proliferation and mesangiolysis without any immune deposits. Tocilizumab suppressed systemic inflammation, resulting in improvement of anasarca and renal dysfunction, but thrombocytopenia persisted and platelet-associated IgG antibody was elevated. Though romiplostim was effective for thrombocytopenia, the patient died of aspiration pneumonia after cerebral hemorrhage...
April 4, 2018: Human Pathology
https://www.readbyqxmd.com/read/29624703/epidemiology-outcome-and-risk-factors-for-infectious-complications-in-myelofibrosis-patients-receiving-ruxolitinib-a-multicenter-study-on-446-patients
#8
Nicola Polverelli, Giuseppe A Palumbo, Gianni Binotto, Elisabetta Abruzzese, Giulia Benevolo, Micaela Bergamaschi, Alessia Tieghi, Massimiliano Bonifacio, Massimo Breccia, Lucia Catani, Mario Tiribelli, Mariella D'Adda, Nicola Sgherza, Alessandro Isidori, Francesco Cavazzini, Bruno Martino, Roberto Latagliata, Monica Crugnola, Florian Heidel, Costanza Bosi, Adalberto Ibatici, Francesco Soci, Domenico Penna, Luigi Scaffidi, Franco Aversa, Roberto M Lemoli, Umberto Vitolo, Antonio Cuneo, Domenico Russo, Michele Cavo, Nicola Vianelli, Francesca Palandri
Infections represent one of the major concerns regarding the utilization of ruxolitinib (RUX) in patients with myelofibrosis. With the aim to investigate epidemiology, outcome and risk factors for infections in RUX-exposed patients, we collected clinical and laboratory data of 446 myelofibrosis patients treated with RUX between June 2011 and November 2016 in 23 European Hematology Centers. After a median RUX exposure of 23.5 months (range, 1-56), 123 patients (28%) experienced 161 infectious events (grades 3-4 32%, fatal 9%), for an incidence rate of 17 cases per 100 pts/y...
April 6, 2018: Hematological Oncology
https://www.readbyqxmd.com/read/29622658/value-of-cytogenetic-abnormalities-in-post-polycythemia-vera-and-post-essential-thrombocythemia-myelofibrosis-a-study-of-the-mysec-project
#9
Barbara Mora, Toni Giorgino, Paola Guglielmelli, Elisa Rumi, Margherita Maffioli, Alessandro Rambaldi, Marianna Caramella, Rami Komrokji, Jason Gotlib, Jean-Jacques Kiladjian, Francisco Cervantes, Timothy Devos, Francesca Palandri, Valerio De Stefano, Marco Ruggeri, Richard T Silver, Giulia Benevolo, Francesco Albano, Chiara Cavalloni, Daniela Barraco, Michele Merli, Daniela Pietra, Rosario Casalone, Tiziano Barbui, Giada Rotunno, Mario Cazzola, Alessandro Maria Vannucchi, Francesco Passamonti
No abstract text is available yet for this article.
April 5, 2018: Haematologica
https://www.readbyqxmd.com/read/29618692/-transfusion-independence-achieved-with-pomalidomide-therapy-in-a-patient-with-primary-myelofibrosis
#10
Yoko Edahiro, Akihiko Gotoh, Tadaaki Inano, Miyuki Tsutsui, Yutaka Tsukune, Hajime Yasuda, Norio Komatsu
Primary myelofibrosis (PMF) is commonly associated with anemia. IMiD® immunomodulatory drugs including thalidomide and lenalidomide have been shown to be effective in improving anemia associated with PMF. However, because of adverse events, their use has been restricted. Herein we report the case of a 67-year-old male patient with transfusion-dependent PMF treated with the immunomodulatory drug pomalidomide in a clinical trial. Significant improvements in anemia and thrombocytopenia were observed with pomalidomide, and the patient recovered from transfusion dependence for 8 months...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29617043/a-novel-type-of-2-base-pair-frameshift-calr-mutation-in-a-patient-with-myeloproliferative-neoplasm
#11
Hyun-Young Kim, Jong-Won Kim, Sun-Hee Kim, Myung Hee Chang, Hee-Jin Kim
Somatic CALR mutations have been identified in the majority of JAK2 mutation-negative essential thrombocythaemia (ET) and primary myelofibrosis. Almost all CALR mutations have been reported to typically generate a +1-base pair (bp) frameshift in the open reading frame. Here, we describe an ET patient with a +2-bp frameshift CALR mutation. A 41-year-old man was admitted because of headache, and diagnosed as JAK2-negative ET. After 4 years, his disease progressed to post-ET myelofibrosis, and CALR mutation analysis demonstrated a +2-bp frameshift CALR mutation caused by two different CALR mutations, c...
April 4, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29616317/outcomes-of-patients-with-myelofibrosis-treated-with-compassionate-use-pacritinib-a-sponsor-independent-international-study
#12
J Mascarenhas, E Virtgaym, M Stal, H Blacklock, A T Gerds, R Mesa, P Ganly, D Snyder, I Tabbara, D Tremblay, E Moshier
Myelofibrosis (MF) is a chronic yet progressive myeloid neoplasm in which only a minority of patients undergo curative therapy, hematopoietic stem cell transplantation. Ruxolitinib, a JAK1/2 inhibitor, is the lone therapy approved for MF, offering a clear symptom and spleen benefit at the expense of treatment-related cytopenias. Pacritinib (PAC), a multi-kinase inhibitor with specificity for JAK2, FLT3, and IRAK1 but sparing JAK1, has demonstrated clinical activity in MF with minimal myelosuppression. Due to an FDA-mandated full clinical hold, the randomized phase 3 PERSIST trials were abruptly stopped and PAC was immediately discontinued for all patients...
April 3, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29611379/gata1-expression-in-bcr-abl1-negative-myeloproliferative-neoplasms
#13
Naery Yang, Sholhui Park, Min Sun Cho, Miae Lee, Ki Sook Hong, Yeung Chul Mun, Chu Myong Seong, Hee Jin Huh, Jungwon Huh
BACKGROUND: This study aimed to determine GATA1 expression levels to better characterize subgroups in BCR/ABL1-negative myeloproliferative neoplasms (MPNs). METHODS: This study enrolled 49 patients diagnosed as having BCR/ABL1-negative MPN on the basis of the 2016 World Health Organization classification : nine polycythemia vera (PV), 17 essential thrombocythemia (ET), 12 prefibrotic primary myelofibrosis (prePMF), and 11 overt primary myelofibrosis (PMF). Relevant clinical and laboratory data were retrieved from the medical records...
July 2018: Annals of Laboratory Medicine
https://www.readbyqxmd.com/read/29597187/combination-therapy-with-ruxolitinib-and-hydroxyurea-for-the-treatment-of-myeloid-predominant-leukocytosis-in-a-patient-with-myelofibrosis
#14
Giovanni Caocci, Silvia Ghiani, Cristina Mocci, Giorgio La Nasa
No abstract text is available yet for this article.
March 29, 2018: Acta Haematologica
https://www.readbyqxmd.com/read/29596070/chronic-myelomonocytic-leukemia-with-fibrosis-is-a-distinct-disease-subset-with-myeloproliferative-features-and-frequent-jak2-p-v617f-mutations
#15
Gur Deniz, Sanam Loghavi, Guillermo Garcia-Manero, Mark Routbort, Rashmi Kanagal-Shamanna, Andres Quesada, Haitham Khogeer, Sherry Pierce, L Jeffrey Medeiros, Hagop Kantarjian, Joseph D Khoury
A subset of patients with chronic myelomonocytic leukemia (CMML) presents with significance myelofibrosis. In myelodysplastic syndromes, significant myelofibrosis has been associated with adverse outcomes and p53 dysregulation. However, in CMML the clinical and molecular correlates of significant myelofibrosis at presentation remain poorly understood. From a cohort of 651 CMML patients, we identified retrospectively 20 (3.1%) cases with moderate to severe reticulin fibrosis (CMML-F) detected at diagnosis, and we compared them to CMML patients without fibrosis (n=631) seen during the same period...
March 28, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29587261/high-frequency-of-copy-neutral-loss-of-heterozygosity-in-patients-with-myelofibrosis
#16
Milton Rego de Paula Junior, Alexandre Nonino, Juliana Minuncio Nascimento, Raphael S Bonadio, Aline Pic-Taylor, Silviene F de Oliveira, Rinaldo Wellerson Pereira, Cintia do Couto Mascarenhas, Juliana Forte Mazzeu
Myelofibrosis is the rarest and most severe type of Philadelphia-negative classical myeloproliferative neoplasms. Although mutually exclusive driver mutations in JAK2, MPL, or CALR that activate JAK-STAT pathway have been related to the pathogenesis of the disease, chromosome abnormalities have also been associated with the phenotype and prognosis of the disease. Here, we report the use of a chromosomal microarray platform consisting of both oligo and SNP probes to improve the detection of chromosome abnormalities in patients with myelofibrosis...
March 22, 2018: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/29575945/current-approaches-to-challenging-scenarios-in-myeloproliferative-neoplasms
#17
Eran Zimran, Ronald Hoffman, Marina Kremyanskaya
The Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia and primary myelofibrosis are clonal hematological malignancies that originate at the level of the hematopoietic stem cell, and are characterized by excessive proliferation of cells belonging to one or more of the myeloid lineages. Central to the pathogenesis of the MPNs is constitutive activation of the JAK/STAT signaling pathway due to a family of driver mutations affecting JAK2, CALR or MPL...
March 29, 2018: Expert Review of Anticancer Therapy
https://www.readbyqxmd.com/read/29574002/treatment-with-jak-inhibitors-in-myelofibrosis-patients-nullifies-the-prognostic-impact-of-unfavorable-cytogenetics
#18
Vincent T Ma, Philip S Boonstra, Kamal Menghrajani, Cecelia Perkins, Krisstina L Gowin, Ruben A Mesa, Jason R Gotlib, Moshe Talpaz
INTRODUCTION: In the era before Janus kinase (JAK) inhibitors, cytogenetic information was used to predict survival in myelofibrosis patients. However, the prognostic value of cytogenetics in the setting of JAK inhibitor therapy remains unknown. PATIENTS AND METHODS: We performed a retrospective analysis of 180 patients with bone marrow biopsy-proven myelofibrosis from 3 US academic medical centers. We fit Cox proportional hazards models for overall survival and transformation-free survival on the bases of 3 factors: JAK inhibitor therapy as a time-dependent covariate, dichotomized cytogenetic status (favorable vs...
March 2, 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29572380/the-identification-of-fibrosis-driving-myofibroblast-precursors-reveals-new-therapeutic-avenues-in-myelofibrosis
#19
Rafael Kramann, Rebekka K Schneider
Myofibroblasts are fibrosis-driving cells and well characterized in solid organ fibrosis but their role and cellular origin in bone marrow fibrosis remained obscure. Recent work has demonstrated that Gli1+ and LepR+ mesenchymal stromal cells (MSC) are progenitors of fibrosis-causing myofibroblasts in the bone marrow. Genetic ablation of Gli1+ MSC or pharmacological targeting Hedghog-Gli signaling ameliorated fibrosis in mouse models of myelofibrosis (MF). Moreover, pharmacologic or genetic intervention in platelet-derived growth factor receptor alpha ( Pdgfra ) signaling in Lepr + stromal cells suppressed their expansion and ameliorated MF...
March 23, 2018: Blood
https://www.readbyqxmd.com/read/29570794/understanding-deregulated-cellular-and-molecular-dynamics-in-the-hematopoietic-stem-cell-niche-to-develop-novel-therapeutics-in-bone-marrow-fibrosis
#20
REVIEW
Hélène F E Gleitz, Rafael Kramann, Rebekka K Schneider
Bone marrow fibrosis is the continuous replacement of blood forming cells in the bone marrow by excessive scar tissue, leading to failure of the body to produce blood cells and ultimately to death. Myofibroblasts are fibrosis-driving cells and well characterized in solid organ fibrosis but their role and cellular origin in bone marrow fibrosis has remained obscure. Recent work has demonstrated that Gli1+ and Leptin Receptor+ mesenchymal stromal cells are progenitors of fibrosis-causing myofibroblasts in the bone marrow...
March 23, 2018: Journal of Pathology
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